The amiloride derivative phenamil attenuates pulmonary vascular remodeling by activating NFAT and the bone morphogenetic protein signaling pathway.

@article{Chan2011TheAD,
  title={The amiloride derivative phenamil attenuates pulmonary vascular remodeling by activating NFAT and the bone morphogenetic protein signaling pathway.},
  author={Mun Chun Chan and Alexandra S. Weisman and Hara Kang and Peter H. Nguyen and Tyler A. Hickman and Samantha V. Mecker and Nicholas S Hill and Giorgio Lagna and Akiko Hata},
  journal={Molecular and cellular biology},
  year={2011},
  volume={31 3},
  pages={
          517-30
        }
}
Pulmonary artery hypertension (PAH) is characterized by elevated pulmonary artery resistance and increased medial thickness due to deregulation of vascular remodeling. Inactivating mutations of the BMPRII gene, which encodes a receptor for bone morphogenetic proteins (BMPs), are identified in ∼60% of familial PAH (FPAH) and ∼30% of idiopathic PAH (IPAH) patients. It has been hypothesized that constitutive reduction in BMP signal by BMPRII mutations may cause abnormal vascular remodeling by… CONTINUE READING
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